Hepatic anaplerotic outflow fluxes are redirected from gluconeogenesis to lactate synthesis in patients with Type 1a glycogen storage disease

Metabolic Engineering

Volumn 11, Issue 3, 2009, Pages 155-162

  Jones, John G ^a   Garcia, Paula ^b   Barosa, Cristina ^a   Delgado, Teresa C ^a   Diogo, Luisa ^b  

^a UNIVERSITY OF COIMBRA   (Portugal)

^b CENTRO HOSPITALAR E UNIVERSITÁRIO DE COIMBRA   (Portugal)

Author keywords

13C NMR; Anaplerosis; Gluconeogenesis; Isotopomer analysis; Pyruvate recycling

Indexed keywords

13C NMR; ANAPLEROSIS; GLUCONEOGENESIS; ISOTOPOMER ANALYSIS; PYRUVATE RECYCLING;

AMINO ACIDS; GLUCOSE; GLYCEROL; ISOMERS; ISOTOPES; NUCLEAR MAGNETIC RESONANCE; NUCLEAR MAGNETIC RESONANCE SPECTROSCOPY; OXIDATION; PLASMAS; RECYCLING;

METABOLISM;

ACETYL COENZYME A; ARYLBUTYRIC ACID DERIVATIVE; CARBON 13; CITRATE SYNTHASE; GLUCOSE; GLUCOSE 6 PHOSPHATE; GLUTAMINE; GLYCEROL; LACTIC ACID; PARACETAMOL; PYRUVIC ACID; STARCH; CARBON; GLUCOSE 6 PHOSPHATASE; LACTIC ACID DERIVATIVE;

ADOLESCENT; ADULT; AMINO ACID BLOOD LEVEL; ARTICLE; BIOPSY; BLOOD SAMPLING; CHILD; CITRIC ACID CYCLE; CLINICAL ARTICLE; CONTROLLED STUDY; FEMALE; GLUCONEOGENESIS; GLUCOSE BLOOD LEVEL; GLYCOGEN STORAGE DISEASE TYPE 1; HUMAN; LACTATE BLOOD LEVEL; LIVER; LIVER CIRCULATION; MALE; NUCLEAR MAGNETIC RESONANCE; PRIORITY JOURNAL; PROTEIN BLOOD LEVEL; SCHOOL CHILD; BLOOD; CHEMISTRY; METABOLISM; PHYSIOLOGY; YOUNG ADULT;

ACETYL COENZYME A; ADOLESCENT; CARBON ISOTOPES; CHILD; CITRIC ACID CYCLE; FEMALE; GLUCONEOGENESIS; GLUCOSE; GLUCOSE-6-PHOSPHATASE; GLUTAMINE; GLYCEROL; GLYCOGEN STORAGE DISEASE TYPE I; HUMANS; LACTATES; LIVER; MALE; PYRUVIC ACID; YOUNG ADULT;

EID: 63949084742     PISSN: 10967176     EISSN: 10967184     Source Type: Journal    
DOI: 10.1016/j.ymben.2009.01.003     Document Type: Article

References (30)

1. Bandsma R.H.J., Smit G.P.A., and Kuipers F. Disturbed lipid metabolism in glycogen storage disease type 1. Eur. J. Pediatr. 161 (2002) S65-S69
2. Bandsma R.H.J., Rake J.P., Visser G., Neese R.A., Hellerstein M.K., van Duyvenvoorde W., Princen H.M.G., Stellaard F., Smit G.P.A., and Kuipers F. Increased lipogenesis and resistance of lipoproteins to oxidative modification in two patients with glycogen storage disease type 1a. J. Pediatr. 140 (2002) 256-260
3. 13C]isocaproate. J. Biol. Chem. 270 (1995) 1509-1514
4. 13C NMR isotopomer analysis of human urine. Anal. Biochem. 312 (2003) 228-234
5. Comte B., Kasumov T., Pierce B.A., Puchowicz M.A., Scott M.E., Dahms W., Kerr D., Nissim I., and Brunengraber H. Identification of phenylbutyrylglutamine, a new metabolite of phenylbutyrate metabolism in humans. J. Mass Spectrom. 37 (2002) 581-590
6. Diraison F., Large V., Brunengraber H., and Beylot M. Non-invasive tracing of liver intermediary metabolism in normal subjects and in moderately hyperglycaemic NIDDM subjects. Evidence against increased gluconeogenesis and hepatic fatty acid oxidation in NIDDM. Diabetologia 41 (1998) 212-220
7. Diraison F., Large V., Maugeais C., Krempf M., and Beylot M. Noninvasive tracing of human liver metabolism: comparison of phenylacetate and apoB-100 to sample glutamine. Am. J. Physiol. 277 (1999) E529-E536
8. 2 in estimating rates of hepatic gluconeogenesis. Am. J. Physiol. 263 (1992) E36-E41
9. Fonseca C.P., Jones J.G., Carvalho R.A., Jeffrey F.M.H., Montezinho L.P., Geraldes C., and Castro M. Tricarboxylic acid cycle inhibition by Li+ in the human neuroblastoma SH-SY5Y cell line: a 13C NMR isotopomer analysis. Neurochem. Int. 47 (2005) 385-393
10. Hellerstein M.K., Greenblatt D.J., and Munro H.N. Glycoconjugates as noninvasive probes of intrahepatic metabolism-pathways of glucose entry into compartmentalized Hepatic UDP-glucose pools during glycogen accumulation. Proc. Natl. Acad. Sci. USA 83 (1986) 7044-7048
11. Jin E.S., Burgess S.C., Merritt M.E., Sherry A.D., and Malloy C.R. Differing mechanisms of hepatic glucose overproduction in triiodothyronine-treated rats vs. Zucker diabetic fatty rats by NMR analysis of plasma glucose. Am. J. Physiol. 288 (2005) E654-E662
12. 13C]propionate, phenylacetate, and acetaminophen. Am. J. Physiol. 275 (1998) E843-E852
13. 3]propionate. FEBS Lett. 412 (1997) 131-137
14. 13C NMR study of gluconeogenesis and TCA cycle flux in humans. Am. J. Physiol. 281 (2001) E848-E851
15. 13C]glucose. Biomed. Environ. Mass Spectrom. 16 (1988) 305-308
16. Kalderon B., Korman S.H., Gutman A., and Lapidot A. Glucose recycling and production in Glycogenosis Type-I and Type-Iii-stable isotope technique study. Am. J. Physiol. 257 (1989) E346-E353
17. 13C-labeled lactate. J. Biol. Chem. 268 (1993) 25509-25521
18. Kien C.L., Horswill C.A., Zipf W.B., McCoy K.S., and Odorisio T. Elevated hepatic glucose production in children with Cystic Fibrosis. Pediatr. Res. 37 (1995) 600-605
19. 14C-labeled propionate metabolism in vivo and estimates of hepatic gluconeogenesis relative to Krebs cycle flux. Am. J. Physiol. 265 (1993) E636-E647
20. Landau B.R., Wahren J., Previs S.F., Ekberg K., Chandramouli V., and Brunengraber H. Glycerol production and utilization in humans: sites and quantitation. Am. J. Physiol. 271 (1996) E1110-E1117
21. Large V., and Beylot M. Modifications of citric acid cycle activity and gluconeogenesis in streptozotocin-induced diabetes and effects of metformin. Diabetes 48 (1999) 1251-1257
22. Large V., Brunengraber H., Odeon M., and Beylot M. Use of labeling pattern of liver glutamate to calculate rates of citric acid cycle and gluconeogenesis. Am. J. Physiol. 272 (1997) E51-E58
23. Magnusson I., Chandramouli V., Schumann W.C., Kumaran K., Wahren J., and Landau B.R. Pentose pathway in human liver. Proc. Natl. Acad. Sci. USA 85 (1988) 4682-4685
24. Magnusson I., Schumann W.C., Bartsch G.E., Chandramouli V., Kumaran K., Wahren J., and Landau B.R. Noninvasive tracing of Krebs cycle metabolism in liver. J. Biol. Chem. 266 (1991) 6975-6984
25. 13C isotope isomers. Am. J. Physiol. 259 (1990) H987-H995
26. Rognstad R., and Katz J. Role of pyruvate kinase in the regulation of gluconeogenesis from l-lactate. J. Biol. Chem. 252 (1977) 1831-1833
27. 13C magnetic resonance spectroscopy. Magn. Reson. Imaging 14 (1996) 1217-1220
28. She P.X., Burgess S.C., Shiota M., Flakoll P., Donahue E.P., Malloy C.R., Sherry A.D., and Magnuson M.A. Mechanisms by which liver-specific PEPCK knockout mice preserve euglycemia during starvation. Diabetes 52 (2003) 1649-1654
29. 13C]glucose study. Am. J. Physiol. 270 (1996) E709-E717
30. Yang D., Beylot M., Agarwal K.C., Soloviev M.V., and Brunengraber H. Assay of the human liver citric acid cycle probe phenylacetylglutamine and of phenylacetate in plasma by gas chromatography-mass spectrometry. Anal. Biochem. 212 (1993) 277-282